CLINICOPATHOLOGIC STUDIES FOR HISTOLOGICAL CLASSIFICATION AND PROGNOSIS OF RHABDOMYOSARCOMA

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1) We reviewed 6 cases of rhabdomyosarcoma as a rare second primary malignancy in children with bilateral retinoblastoma after irradiation treatment. We have concluded that knowledge of the occurrence of rhabdomyosarcoma and appropriate immunohistochemical study are helpful for avoiding a misdiagnosis of recurrent retinoblastoma or Ewing's sarcoma when encountering these patients who developed second small round cell neoplasms.2) We studied immunoreactivity for E-cadherin, the major cadherin of epithelial cells, in 77 cases of a variety of bone and soft tissue sarcomas. We found that E-cadherin was expressed in certain kinds of soft tissue sarcomas, especially those with epithelioid features, suggesting that E-cadherin plays a role in the constitution of their architecture.3) We conducted clinicopathologic study for 24 cases of extrathoracic solitary fibrous tumor that should be differentiated from spindle cell rhabdomyosarcoma, and found their histological variability and potentially aggressive behavior.4) We investigated the prognostic relevance of a histological grading system based on the assessment of proliferative activity in 95 cases of adult soft-tissue sarcomas including 3 rhabdomyosarcomas of the extremities, trunk, head, and neck. An objective histological grading system using ki-67 (MIB-1) was concluded to be more accurate in accessing ability to metastasize, which was strongly correlate with overall survival.